A 28-year-old man without tuberous sclerosis, who complained of pollakisuria, consulted to our hospital for a left renal mass. Abdominal computed tomography revealed a solid mass without a lipid component, 10 cm in diameter, in the left kidney and with regional lymphadenopathy. Renal arteriography showed a hypervascular mass, demonstrating multiple tumor stains and aneurysms. Left radical nephrectomy and perihilar lymph node dissection were performed for an anticipated diagnosis of malignant tumor in November 2001. The histopathological diagnosis was an angiomyolipoma with lymph node involvement. Immunostaining for myogen markers was positive in the renal mass and lymph node tumors. He was free from disease ten months after surgery.